• Rheumatoid arthritis (RA) is a chronic, system autoimmune disorder that presents primarily as a polyarticular inflammatory arthritis but may also manifest with extraarticular features.
• If left uncontrolled, the disease leads to destruction of joints due to erosion of cartilage and bone, leading to joint deformities that begin in the periphery and progress to proximal joints.
• The diagnosis of RA is clinical, and should be considered in patients with symmetrical polyarthralgia, joint stiffness, and/or synovitis lasting more than 6 weeks.
• Although the disease itself is not fatal, complications of the disease make it the most common rheumatic disease requiring ICU admission.
• Complications include: atlanto-axial subluxation, cricoarytenoid arthritis, interstitial pulmonary fibrosis, accelerated coronary atherosclerosis, septic arthritis, Felty’s syndrome (triad of RA, splenomegaly and neutropenia), renal failure, and GI hemorrhage amongst many others.

Initial Clinical Presentation

• Insidious in onset with predominant symptoms being pain, stiffness, and swelling of many joints.
• Early typical presentations involve symmetric synovial joint involvement affecting the MCP and PIP joints with sparing of the DIP.
• Morning stiffness or stiffness after a prolonged period of inactivity.

Evaluation

1. History

• Joint involvement, characteristics.
• Morning stiffness (often > 30 min) that usually improves with activity.

2. Physical Exam

• Articular manifestations
  • Symmetrical, polyarticular arthritis often involving MCP, PIP, wrists, knees, but sparing the DIP, CMC, and axial skeleton (except for C-spine).
  • Joint deformities: Swan neck deformity, Boutonniere deformity, hitchhiker thumb, ulnar deviation of fingers.
• Extraarticular manifestations
  • Constitutional symptoms: low-grade fever, myalgia, malaise, fatigue, weight loss, night sweats.
  • Rheumatoid nodules.
  • Lungs: pleuritis, pleural effusions, interstitial lung disease.
  • Eye: keratoconjunctivitis, scleritis, episcleritis.
  • Hematological: anemia, neutropenia, splenomegaly, lymphoma.
  • Heart: pericarditis, myocarditis.
  • Vascular: vasculitis, purpura, livedo reticularis.

3. Investigations

• CRP or ESR.
• CBC.
• Thyroid function tests – autoimmune thyroid disorder is common in patients with RA.
• Serology: ANA, ACPA, RF – note serological studies may be negative (e.g., seronegative RA).
• Synovial fluid analysis – if suspicious of septic arthritis or to exclude other diagnoses.

4. Imaging

• X-ray: recommended as the initial test to assess joint disease severity.
• Ultrasound: utility in assessing synovitis and joint effusion.
• MRI: consider if cervical spine involvement suspected (e.g., atlantoaxial subluxation).

Diagnostic Criteria

The 2010 American College of Rheumatology/European League Against Rheumatism Classification Criteria for RA.

ED Treatment of Acute Flares

Temporary (< 3 months) symptomatic treatment with glucocorticoids and/or NSAIDs.

First line: NSAIDs and selective COX-2 inhibitors

• Ibuprofen, diclofenac, celecoxib.
• No agents have superior efficacy.
• Trial one for 4 weeks at high doses, switch to a different class for 4 weeks.

Second line: Glucocorticoids

• Methylprednisolone 2-32 mg PO daily, 1 to 4-week taper.
• Prednisone 5-10 mg PO daily, 4-week taper.

Long-term pharmacological treatment

Disease modifying anti-rheumatic drugs (DMARDs)

• Methotrexate, hydroxychloroquine, sulfasalazine, tofacitinib.

Airway

RA causes chronic structural changes to the cervical spine and airway which can increase risk of airway complications.

Atlanto-axial subluxation

• Clinical features: new occipital pain, extremity paresthesias, sensory disturbances, weakness of upper extremities, vertigo, focal neurological deficits.
• Airway management
  • Avoid neck hyperextension, even in the absence of symptoms.
  • In-line mobilization should be maintained, and fiberoptics or video laryngoscopy.
  • Ensure preparation for emergent cricothyrotomy.
• NOTE: DO NOT passively flex or hyperextend the neck of patients with RA as subluxation may be clinically silent.

Cricoarytenoid arthritis

• Clinical features: hoarseness, foreign body sensation, dysphagia, vocal changes, stridor, cough, wheeze, tenderness over cartilaginous structures.
• Airway management
  • Ensure preparation for difficult airway and emergent cricothyrotomy.
• Treatment
  • High-dose IV methylprednisolone 250-500 mg.

Tracheomalacia

• Airway management
  • Smaller than normal endotracheal tube size to avoid cartilaginous destruction.

Pulmonary

Interstitial lung disease and pulmonary fibrosis

• Clinical features: insidious onset cough and exertional dyspnea. Severe cases may lead to pulmonary hypertension and cor pulmonale.
• Imaging shows diffuse infiltrates and PFTs reveal restrictive lung pattern.
• Treatment
  • High-dose IV methylprednisolone 1 mg/kg/day for 3 days followed by oral prednisolone 1 mg/kg/day until symptoms improve.

Pleuritis

• Clinical features: dyspnea, pleuritic chest pain, pleural effusions, or friction rub.
• Diagnosis: diagnostic and therapeutic thoracentesis to prevent scarring or fibrosis.

Alveolar hemorrhage

• Clinical features: massive hemoptysis.
• Management: early bronchoscopy, high-dose IV methylprednisolone up to 500 mg q6h, and consideration of tranexamic acid.

Cardiovascular

Accelerated coronary atherosclerosis

• RA patients are at a three-fold increased risk for myocardial infarctions compared to the general population.
• These patients often present with atypical chest pain that is attributed to arthritis, leading to missed MIs and significant morbidity and mortality.

Musculoskeletal

Septic arthritis

• Septic arthritis can easily missed as symptoms are attributed to RA flare.
• Diagnostic arthrocentesis should be performed if there is clinical suspicion.

Hematological

Felty syndrome

• Triad: neutropenia, splenomegaly, and rheumatoid arthritis.
• Patients require thorough evaluation for infection given risk for recurrent and fulminant bacterial infections.

Renal

Renal failure

• RA patients are at increased risk of renal failure secondary to nephritis, rhabdomyolysis, renal vein thrombosis, and fulminant renal failure.
• New onset acute kidney injury should prompt further investigation and early involvement of nephrology and rheumatology.
• Diagnostics include urinalysis and electrolytes however, definitive diagnosis is made via renal biopsy.

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